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2017 ; 56
(5
): 557-562
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Splenic Marginal Zone Lymphoma with Acquired von Willebrand Syndrome Diagnosed
via Splenic Bleeding
#MMPMID28250305
Komeno Y
; Shibuya N
; Uryu H
; Yamada H
; Toda T
; Shibasaki M
; Kunishima S
; Iihara K
; Ryu T
Intern Med
2017[]; 56
(5
): 557-562
PMID28250305
show ga
An 85-year-old woman underwent emergent splenectomy due to left abdominal pain
and active bleeding in a massively enlarged spleen. The histological diagnosis
was splenic marginal zone lymphoma (SMZL). A prolonged activated partial
thromboplastin time (APTT) was noted, and additional tests led to the diagnosis
of type 2A-like acquired von Willebrand syndrome (AVWS). An APTT cross mixing
test ruled out the presence of inhibitors. She received eight courses of
rituximab monotherapy. The coagulation data showed no improvement, possibly
because the lymphoma showed a poor response to the treatment. AVWS rarely causes
bleeding in solid organs. This is the first case of SMZL with AVWS diagnosed via
splenic bleeding.